A Child With Primary Sjögren Syndrome and a Review of the Literature
- 6 June 2007
- journal article
- case report
- Published by SAGE Publications in Clinical Pediatrics
- Vol. 46 (8), 738-742
- https://doi.org/10.1177/0009922807301945
Abstract
Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and purpura episodes since 9 years of age. During her 3-week hospitalization, 2 episodes of parotid swelling occurred, which both resolved in 48 hours. Ultrasonography and magnetic resonance images of parotid glands showed parenchymal inhomogeneity related to adipose degeneration and nodular pattern. Investigations showed elevated erythrocyte sedimentation rate, the presence of hypergammaglobulinemia, positive antinuclear antibody, and elevated rheumatoid factor, anti—Sjögren syndrome antigen A, and anti—Sjögren syndrome antigen B. Histopathologic examination of labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. She was diagnosed as having pSS. Recurrent parotid swelling is a more characteristic feature of disease in children, and this finding should alert the clinician to the possible diagnosis of pSS.Keywords
This publication has 16 references indexed in Scilit:
- Sjögren's syndromeThe Lancet, 2005
- Primary sjögren syndrome in childhood: report of a case and review of the literatureOral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology, 2003
- Primary Sjögren syndrome in the paediatric age: a multicentre surveyEuropean Journal of Pediatrics, 2003
- Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus GroupAnnals Of The Rheumatic Diseases, 2002
- Primary and secondary Sjögren's syndrome in children - a comparative studyClinical Oral Investigations, 2000
- Complications of childhood Sjögren syndromeEuropean Journal of Pediatrics, 1996
- Sjögren's syndrome with membranous glomerulonephritis detected by urine screening of schoolchildrenPediatrics International, 1996
- Central nervous system disease in a child with primary Sjögren syndromeThe Journal of Pediatrics, 1995
- Anti-Ro/SSA-associated annular erythema in childhoodBritish Journal of Dermatology, 1995
- Primary Sjogren's syndrome and rampant dental caries in a 5‐year‐old childInternational Journal of Paediatric Dentistry, 1995